肺泡蛋白沉积症影像学分析

Radiological features of pulmonary alveolar proteinosis

目的 探讨肺泡蛋白沉积症 (pulmonaryalveolarproteinosis ,PAP)的X线、CT影像学特征。 方法 回顾性分析6例经病理证实的PAP患者的胸部X线和CT表现。结果 PAPX线表现为两肺对称或不对称分布磨玻璃样影和实变影 ,自肺门向外放射 ,可形成“蝶翼征”。胸部CT呈多样化改变 :①从弥散分布的模糊结节影到弥漫性斑片状磨玻璃影与气腔实变影 (6例 ) ,呈地图样分布 (5例 ) ;②蝶翼征 (2例 ) ;③支气管充气征 (3例 ) ;④铺路石征 (5例 )。结论 肺泡蛋白沉积症CT表现有一定特征性 ;纤支镜肺活检是本病确诊的主要依据。

Objective To probe the X ray and CT findings of pulmonary alveolar proteinosis (PAP). Methods The X ray and CT findings of 6 cases with pathologically proven PAP were retrospectively analyzed. Results Symmetry or dissymmetry ground glass opacities and air space opacities were found in X ray films, distributed on two lungs,radiated from hila to sides, forming butter fly signs. On HRCT, a variety of abnormalities were found as follows:①diffuse ill defined nodular shadows to diffuse patchy cloudy shadows,ground glass and consolidation opacities ( n =6), distributed geographically ( n =5), ②butter fly signs ( n =2), ③air bronchogram sign ( n =3), and ④”crazy paving” ( n =5), the characteristic appearance of typical PAP. Conclusion The typical CT features of PAP are helpful to the diagnosis of PAP. Transbronchial lung biopsy is still the main approach to the final diagnosis of PAP.